RESUMO
Skull base chordomas are rare neoplasms arising from the notochord. Although histologically benign, these tumors are locally aggressive and present significant management challenges. There are some studies on chordoma cases but there was no study about Iranian cases. In this study we evaluated the location, age and gender of the patients with Chordoma in two referral centers in Tehran. A database of patients with chordoma tumors referred to two centers [Shariati and Imam Hospitals, Tehran] from 2001 to 2011 was retrospectively reviewed. In our subjects tumors affect men nearly twice as frequently as women, and they are most commonly diagnosed in middle-aged [mean age was 50.6]. Tumors typically occur in the axial skeleton and have a tendency for the spheno-occipital region of the skull base and sacral region. In adults 33.3% of chordomas involve the sacrococcygeal region, 53% occured at the base of the skull near the spheno-occipital area, and near 14% were found in the vertebral column. The cranial nerves mostly affected were abducens, oculomotor and trochlear, with some overlaps. All patients were treated with surgery and some cases referred for gamma-knife radiosurgery [GKS]. Findings of this study showed more involvement of males compare to females; that is different from other studies, however, few studies reported more male to female ratio. Despite the progress in current surgical techniques and some encouraging results with the use of targeted therapy, disease control and long-term prognosis of patients are still poor